Internal auditory canal stenosis in congenital sensorineural hearing loss

OBJECTIVE: Sensorineural hearing loss (SNHL) due to internal auditory canal (IAC) stenosis with hypoplasia of the cochleovestibular nerve is a rare disorder. The diagnosis of the IAC stenosis requires both high resolution computed tomography scan (HRCT) and magnetic resonance imaging (MRI). METHODS: A retrospective review over 6 years in an academic tertiary referral center was performed. RESULTS: Six patients with congenital SNHL were diagnosed with congenital IAC stenosis. Four had unilateral and two had bilateral IAC stenosis after imaging. MRI showed hypoplastic vestibulocochlear nerve in all cases. CONCLUSIONS: This paper highlights the importance of imaging in diagnosing IAC stenosis and detecting the presence of cochleovestibular nerve in cases of congenital SNHL.